Immunodeficiency Disorders Lecture Powerpoint Flashcards
8/26/19
6 signs that immune system may not be functioning properly
1) 8 or more new ear infections in a year
2) two or more serious sinus infections in a year (particularly in kids)
3) two or more months on persistent antibiotics without relief
4) failure to thrive
5) oral thrush present after 1 year of age
6) recurrent need for IV antibiotics to clear infections
CMV opportunistic infeection
Causes eye disease that can lead to blindness in immunocompromised host as well as infants thru maternal delivery
Herpes simplex virus opportunistic infection
Very severe, recurrent, symptomatic herpes outbreaks can imply immunocompromised host
Mycobacterium avium complex opportunistic infection
Bacterial infection causing recurring fevers and malaise in immunocompromised host
Pneumocystis pneumonia (PCP) opportunistic infection
Fungal infection that can cause fatal pneumonia, fairly common in immunocompromised hosts
Toxoplasmosis
Protozoal infection of the brain often sourced from cat feces in immunocompromised hosts
Primary vs 2ndary immunodeficiencies
Primary are not attributable to other disease processes, 2ndary are such as DM, cushings, chemo
Significant molecule in the production of neutrophils and macrophages
Colony stimulating factor (G-CSF)
Congenital neutropenia (what type is it, what should you know about it)
Production disorder, rare, caused by an ELANE gene mutation
Cyclic neutropenia (what type is it, what should you know about it)
Production disorder, monthly rise and fall in wbc being produced, might see patient getting sick during those times but rarely impacts life significanly
Chediak Higashi syndrome(what type is it, what should you know about it)
Production disorder, autosomal recessive disorder resulting in partial albinisim, impaired phagolysosome production resulting in large granules, frequent bacterial infections, and peripheral neuropathy later in life
4 types of disorders around phagocytes
1) production
2) maturation
3) increased destruction
4) autoimmune
Maturation disorders of phagocytes (3)
- nutritional B12/folate deficiency
- myelodysplastic syndromes
- viral infections
Disorders of increased destruction of phagocytes (3)
- Antiboties
- Vaccines
- post infection disorders
Autoimmune disorders of phagocytes (4)
- Felty syndrome
- systemic lupus erythematosus
- rheumatoid arthritis
- transfusion reaction
Felty syndrome and the triad for diagnosis
Mostly assymptomatic, some develop serious and life threatening infections secondary to granulocytopenia
Rheumatoid arthritis, splenomegaly, neutropenia
Glucocorticosteroids positive and negative effects on the neutrophil
Positive - enhance production and mobilize marginated (peripheral pool ) into circulation
Negative - reduce adherence capacity and chemotactic properties